What is a pharanegyal flap?

Just a little Anatomy class for today! For those of you who are curious here is a description and explanation of Grace's surgery.

A little history- She was in distress at birth from inhaling meconium in-utero which resulted in her being born with chemical pneumonia. She also had very low muscle tone which suggested there would be other issues. Then the adventure began! Long story but in summary, she was very mysterious for years and eventually at 5 was diagnosed with a rare genetic syndrome.

One of the things noticed just after birth was that although she would nurse or take a bottle, she wasn't able to get anything out of it. That is how we discovered she had a cleft in the muscles of her palate. So although her palate looked normal, the muscles underneath were not connected. At 6 months old, they were able to surgically connect muscles in her soft palate. Then with LOTS of therapy she was eventually able to use them with some degree of efficiency . Those muscles help with swallowing and speech. The creation of the pharyngeal flap has been suggested since she was 3 but because of her other issues and her small size, they kept delaying it. This year we begged for them to do something. We went with the saying, "if there is a will, there is a way". They agreed that the window of opportunity was closing for her to successfully correct the speech patterns she has created in an effort to compensate for the defect. So they began to think out of the box. Her anatomy did not allow her to make many of the sounds we use in communication. For example, prior to the flap if she were to blow a whistle nothing happened. If she pinched her nose closed though, she could blow it and make a sound. So in sounds that need pressure the air was escaping her nose. Which results in her sounding like she is talking while yawning. This flap changes the anatomy and gives her the ability to make those sounds. However, the muscles have to strength and adapt to it. It also involves a lot of motor planning but she now has the potential to speak clearer. We will continue to invest in LOTS of speech therapy- man, how I wish there was a return on investment there! The solution the team proposed, after our appointment of desperation, was to do the flap BUT only if they secured the airway with a trach. And that is how we got the flap and the trach.

A huge misconception, and frankly prejudice, is that if you can't speak clearly then you must be retarded. This was one of the main reasons we were so adamant about doing this for Grace. Although signing is an excellent alternative to verbal speech Grace has poor fine motor skills and so even that would have to be approximated. We have tried communication devices too but Grace truly has a desire to communicate with her words. For years she thought it was everyone else's hearing that made them unable to understand her! Once she realized it was her and not the listener she was already sold on verbal communication. We have been very intentional about telling her that although her words are difficult to understand she still has very good things to say and her thoughts and ideas are great and well worth explaining! Granted, it takes a lot of effort as a listener to understand Grace, but it is well worth it. She is very funny and intriguing. The goal of this surgery is that it will be easier for her and the listener and she will be able to communicate effectively with us and the average Joe.

So if you like the gory details this will explain how they did it...

They cut a piece of tissue from the back of her throat and and this is the flap. They then cut open her soft palate back by the uvula, separated it inserted the flap, then closed it back up. So it is sort of like a bridge across her airway, from the back of her throat to her palate. This is not normal anatomy it is a compensation for not having normal palate length, tissue and muscles in her palate. The flap covers a portion of her airway and thus the trach. Before the surgery and in normal anatomy the airway is open and now with the flap is taking the middle part of it. For instance instead of a circle it is now a circle with a thick line through it. Her breathing from her nose will come from the sides of the original airway and from her mouth rather than a wide open airway from her nose. This takes her brain learning how to adjust. While awake that is rather natural but in sleep with the body and mind relaxed the risk is that the brain doesn't adjust it's breathing and she stops breathing. The trach is an insurance for her, not her main airway but when asleep she uses it to breathe until her brain adjust to her new anatomy. Temporary but necessary. There you have it! Rather useless information for most but now you have a new word for Scrabble...pharyngeal.